• Korean Journal of Adult Nursing
• /
• v.20 no.2
• /
• pp.341-352
• /
• 2008

Purpose: The purpose of this study was to identify relatively important predictors of quality of life (QOL) of HSCT recipients among client's characteristics(age, gender, family income, religiosity), HSCT-related characteristics(time since HSCT, type of HSCT, decision maker of HSCT) and social support. Methods: Eighty two participants who had a HSCT were recruited for the study. Data were analyzed by descriptive analysis, pearson's correlation, ANOVA and stepwise multiple regression using SPSS for Window(version 12.0) program to answer the research questions. Results: Family income, time since HSCT and religiosity explained 23.8% of the variance in the QOL of HSCT recipients. HSCT recipients who had higher family income, longer time past since HSCT, and more religious tend to have higher quality of life. Conclusion: Based on the findings of this study, we could know that the HSCT recipients need certain amount of time to recover their QOL after HSCT. Opportunities of reemployment and religious support should be considered when we develop intervention program for HSCT recipients.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.14 no.1
• /
• pp.29-36
• /
• 2014

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic diseases caused by deficiency of lysosomal enzymes. MPSs are clinically heterogeneous and characterized by progressive deterioration in visceral, skeletal and neurological functions. The aim of this article is to review the treatment of MPSs, the unmet needs of current treatments and vision for the future including recent clinical trials. Until recently, supportive care was the only option available for the management of MPSs. Hematopoietic stem cell transplantation (HSCT), another potentially curative treatment, is not routinely advocated in clinical practice due to its high risk profile and lack of evidence for efficacy. From the early 2000s, enzyme replacement therapy (ERT) was approved and available for the treatment of MPS I, II and VI. ERT is effective for the treatment of many somatic symptoms, particularly walking ability and respiratory function, and remains the mainstay of MPS treatment. However, no benefit was found in the neurological symptoms because the enzymes do not readily cross the blood-brain barrier (BBB). In recent years, intrathecal (IT) ERT, substrate reduction therapy (SRT) and gene therapy have been rapidly gaining greater recognition as potential therapeutic avenues. Although still under investigation, IT ERT, SRT and gene therapy are promising MPS treatments that may prevent the neurodegeneration not improved by ERT.

• Journal of mucopolysaccharidosis and rare diseases
• /
• v.3 no.2
• /
• pp.37-40
• /
• 2017

Mucopolysaccharidosis (MPS) is caused by accumulation of the glycosaminoglycans in all tissues due to decreased activity of the lysosomal enzyme. Patients exhibit multisystemic signs and symptoms in a chronic and progressive manner, especially with changes in the skeleton, cardiopulmonary system, central nervous system, cornea, skin, liver, and spleen. In the past, treatment of MPS was limited to enzyme replacement therapy (ERT). The outcome for affected patients improved with the introduction of new technologies as hematopoietic stem cell transplantation, relegated to specific situations after ERT became available. Intrathecal ERT may be considered in situations of high neurosurgical risk but still it is experimental in humans. New insights on the pathophysiology of MPS disorders are leading to alternative therapeutic approaches, as gene therapy, inflammatory response modulators and substrate reduction therapy. In this paper, we will highlight the recent novel treatment and clinical trials for MPS and discuss with the goal of fostering an understanding of this field.

• Journal of Korean Critical Care Nursing
• /
• v.12 no.1
• /
• pp.46-56
• /
• 2019

Purpose : The purpose of this study was to identify minimum data sets for oral mucous integrity-related documentation and to analyze nursing records for oral care. Methods: To identify minimum data sets for oral status, the authors reviewed 26 assessment tools and a practical guideline for oral care. The content validity of the minimum data sets was assessed by three nurse specialists. To map the minimum data sets to nursing records, the authors examined 107 nursing records derived from 44 patients who received chemotherapy or hematopoietic stem cell transplantation in one tertiary hospital. Results: The minimum data sets were 10 elements such as location, mucositis grade, pain, hygiene, dysphagia, exudate, inflammation, difficulty speaking, and moisture. Inflammation contained two value sets: type and color. Mucositis grade, pain, dysphagia and inflammation were recorded well, accounting for a complete mapping rate of 100%. Hygiene (100%) was incompletely mapped, and there were no records for exudate (83.2%), difficulty speaking (99.1%), or moisture (88.8%). Conclusion: This study found that nursing records on oral mucous integrity were not sufficient and could be improved by adopting minimum data sets as identified in this study.

• Child Health Nursing Research
• /
• v.29 no.4
• /
• pp.280-289
• /
• 2023

Purpose: This study investigated weight status in survivors of childhood acute lymphocytic leukemia (ALL) and identified related factors. Methods: A retrospective review of the electronic medical records of survivors of childhood ALL (n=230) was conducted. We analyzed the survivors' characteristics, including sex, age, weight status at diagnosis, central nervous system involvement, risk classification, length of treatment, radiation therapy, and hematopoietic stem cell transplantation. Analysis of variance and the chi-squared test were applied to investigate influencing factors. Results: The weight status distribution was as follows: 23 individuals (10.0%) were classified as underweight, 151 individuals (65.7%) were healthy weight, and 56 individuals (24.3%) were overweight/obese. Age at diagnosis (F=10.03, p<.001), weight status at diagnosis (x²=43.41, p<.001), and risk classification (F=10.98, p=0.027) showed significant differences among the weight status groups. Survivors who were older at diagnosis and those in the very high-risk category had a higher likelihood of experiencing underweight status during their survivorship, while survivors who were overweight/obese at diagnosis were more likely to remain overweight/obese at the time of survival. Conclusion: Considering the potential health implications related to an unhealthy weight status in survivors of ALL, it is imperative to undertake early identification and implement interventions for at-risk individuals.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.50 no.4
• /
• pp.421-433
• /
• 2023

This study was to examine the developmental dental abnormalities in childhood cancer survivors. Risk factors were assessed for 125 children with radiographic data through a retrospective analysis of medical records and panoramic images. 68.0% of childhood cancer survivors exhibited at least one dental abnormality. The types of abnormalities varied depending on the age at cancer diagnosis and treatment intensity, ranging from microdontia (43.2%), to abnormal root development (39.2%) and tooth agenesis (33.6%). Logistic regression analysis demonstrated that a young age at diagnosis (under 3 years), the use of heavy metal agents, a history of hematopoietic stem cell transplantation (HSCT), and combination treatment of chemotherapy, radiation therapy, and HSCT were associated with a significantly higher risk for overall dental abnormalities. The increased risk ratios were 6.00, 3.06, 3.22, and 7.87, respectively (p < 0.05). The results of this study will predict dental abnormality in permanent dentition according to the diagnosis age and treatment method of childhood cancer.

• Proceedings of the Korean Society of Developmental Biology Conference
• /
• 2003.10a
• /
• pp.71-71
• /
• 2003

Human umbilical cord blood cells(HUCBC) are rich in mesenchymal progenitor cells, endothelial cell precursors and hematopoietic cells. HUCBC have been used as a source of transplantable stem and progenitor cells. However, little is known about survival and development of HUCBC transplantation in the CNS. Estrogen has a neuroprotective potential against oxidative stress-induced cell death so has an effect on reducing infarct size of ischemic brain. We investigated the potential use of HUCBC as donor cells and tested whether estrogen mediates intravenously infused HUCBC enter and survive in ischemic brain. PKH26 labeled mononuclear fraction of HUCBC were injected into the tail vein of ischemic OVX rat brain with or without $17\beta$-estradiol valerate(EV). Under fluorescence microscopy, labeled cells were observed in the brain section. Significantly more cells were found in the ischemic brain than in the non-ischemic brain. HUCBC transplanted into ischemic brain could migrate and survive. Some of cells have shown neuronal like cells in hippocampus, striatum and cortex tissues. These result suggest that estrogen reduces ischemic damage and increases the migration of human umbilical cord blood cells. This Study was supported by the Korea Science and Engineering Foundation(KOSEF) though the Biohealth Products Research Center(BPRC), Inje University, Korea.

• Natural Product Sciences
• /
• v.29 no.1
• /
• pp.42-49
• /
• 2023

This study investigated the effect of ethanol extracts of horsetail, alfalfa, ortie, chêne and aleppo oak on blood coagulation in vitro. Extraction was performed by the maceration method. Extracts were mixed with platelet and plasma, then prothrombin time (PT), activated partial thromboplastin time (APTT) and platelet aggregation tests were conducted. Alfalfa extract had a dose-dependent effect on the PT. Ortie, and horsetail, reduced the PT significantly compared to control group. Alfalfa, horsetail, and ortie reduced the APTT, but their effect was insignificant compared to the control group. The pooled extract showed the highest effect compared to the single extracts in a dose-dependent manner. Horsetail and alfalfa induced platelet aggregation in response to arachidonic acid but not in response to collagen. In the case of ortie, no aggregation occurred regarding the arachidonic acid, and incomplete was observed in response to collagen. Interestingly, blood clotting occurred immediately after adding the chêne, aleppo oak and the pooled extract, and therefore platelet poor plasma (PPP) and platelet rich plasma (PRP) became jelly. Generally, chêne and aleppo oak, as well as pooled extract, were more effective in inducing both primary and secondary coagulation pathways via shortening the PT and APTT, and induction of platelet aggregation.

• Radiation Oncology Journal
• /
• v.35 no.3
• /
• pp.257-267
• /
• 2017

Purpose: Pulmonary toxicities, including infectious pneumonia (IP) and idiopathic pneumonia syndrome (IPS), are serious side effects of total body irradiation (TBI) used for myeloablative conditioning. This study aimed to evaluate clinical factors associated with IP and IPS following TBI. Materials and Methods: Fifty-eight patients with hematologic malignancies who underwent TBI before allogeneic hematopoietic stem cell transplantation between 2005 and 2014 were reviewed. Most patients (91%) received 12 Gy in 1.5 Gy fractions twice a day. Pulmonary toxicities were diagnosed based on either radiographic evidence or reduced pulmonary function, and were subdivided into IP and IPS based on the presence or absence of concurrent infection. Results: Pulmonary toxicities developed in 36 patients (62%); 16 (28%) had IP and 20 (34%) had IPS. IP was significantly associated with increased treatment-related mortality (p = 0.028) and decreased survival (p = 0.039). Multivariate analysis revealed that the risk of developing IPS was significantly higher in patients who received stem cells from a matched unrelated donor than from a matched sibling donor (p = 0.021; hazard ratio [HR] = 12.67; 95% confidence interval [CI], 1.46-110.30). Combining other conditioning agents with cyclophosphamide produced a higher tendency to develop IP (p = 0.064; HR = 6.19; 95% CI, 0.90-42.56). Conclusion: IP and IPS involve different risk factors and distinct pathogeneses that should be considered when planning treatments before and after TBI.

• Pediatric Infection and Vaccine
• /
• v.21 no.2
• /
• pp.81-95
• /
• 2014

Purpose: Hematopoietic cell transplantation (HCT) recipients are vulnerable to invasive infection by Haemophilus influenzae type b (Hib) and Streptococcus pneumoniae (Sp). This study was performed to evaluate immune responses after Hib and Sp vaccination in Korean pediatric HCT recipients. Methods: Patients were prospectively enrolled at Samsung Medical Center during 2009-2011. ELISA tests to detect anti-PRP IgG antibody and antibodies to Sp serotypes 4, 6B, 9V, 14, 18C, 19F, and 23F were performed at the Center for Vaccine Evaluation and Study, Ewha Medical Research Institute. Results: Ten patients (two allogeneic, eight autologous recipients) with median age 5.4 years (range 2.7-12.2 years) were enrolled. Before Hib vaccination, 60% of patients' anti-PRP IgG titers were below $0.15{\mu}g/mL$. After vaccination, 100% of patients' anti-PRP IgG titers increased above $0.15{\mu}g/mL$ (cut-off value for detection) and $1.0{\mu}g/mL$ (cut-off value for seroprotection). For pneumococcus, in 2-5 year-old patients, pre-vaccination geometric mean concentrations (GMCs) of IgG for six serotypes (4, 6B, 9V, 14, 18C, and 23F) were below $0.35{\mu}g/mL$ and at 5 months post-vaccination GMCs of IgG for all seven serotypes increased to above $0.35{\mu}g/mL$. In patients older than 5 years, pre-vaccination GMCs of IgG for four serotypes (4, 9V, 14, and 23F) were below $0.35{\mu}g/mL$ and at 3 months post-vaccination GMCs of IgG for all seven serotypes increased to above $0.35{\mu}g/mL$. Conclusion: Most HCT recipients had low or no protective antibodies to Hib and Sp before vaccination, but showed good immune responses to protective levels after vaccination.